Rabbit Anti-ZIC3/FITC Conjugated antibody

Home > Product > Antibody > Rabbit Anti-ZIC3/FITC Conjugated antibody

Heterotaxy 1; HTX; HTX1; ZIC 3; Zic family member 3 (odd paired Drosophila homolog heterotaxy 1); Zic family member 3; Zic3; ZIC3_HUMAN; Zinc finger protein 203; Zinc finger protein of the cerebellum 3; Zinc finger protein ZIC 3 (Zinc finger protein of th

Cat:

SL11608R-FITC

Species Reactivity：

Mouse,Rat,(predicted: Human,Chicken,Dog,Pig,Cow,Sheep,)

Immunogen：

KLH conjugated synthetic peptide derived from human ZIC3 (331-410aa)

Format：

Lyophilized or Liquid

Storage instructions：

Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

ICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

51kDa

prev next

Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
Feedback Wall

background:
Zic3 is a C2H2 zinc finger transcription factor that establishes a proper left-right axis and midline neural patterning during early development of the vertebrate embryo. Mutations in this gene cause X-linked visceral heterotaxy, which includes congenital heart disease and left-right axis defects in organs. Zic3 mutations in the zinc finger DNA binding domain and in the N-terminal domain result in loss of reporter gene transactivation, and mutations between amino acids 253-323 of the Zic3 protein causes aberrant cytoplasmic localization rather than the wild type nuclear localization.

Function:
Acts as transcriptional activator. Required in the earliest stages in both axial midline development and left-right (LR) asymmetry specification. Binds to the minimal GLI-consensus sequence 5'-GGGTGGTSLC3'.

Subcellular Location:
Nucleus. Cytoplasm. Localizes in the cytoplasm in presence of MDFIC overexpression (By similarity). Translocation to the nucleus requires KPNA1 or KPNA6.

DISEASE:
Defects in ZIC3 are the cause of visceral heterotaxy X-linked type 1 (HTX1) [MIM:306955]. A form of visceral heterotaxy, a complex disorder due to disruption of the normal left-right asymmetry of the thoracoabdominal organs. It results in an abnormal arrangement of visceral organs, and a wide variety of congenital defects. Clinical features of visceral heterotaxy X-linked type 1 include dextrocardia, corrected transposition of great arteries, ventricular septal defect, patent ductus arteriosus, pulmonic stenosis, situs inversus viscerum, and asplenia and/or polysplenia.
Defects in ZIC3 are a cause of VACTERL association X-linked with or without hydrocephalus (VACTERLX) [MIM:314390]. A syndrome characterized by vertebral anomalies, anal atresia, cardiac malformations, tracheoesophageal fistula, renal anomalies (urethral atresia with hydronephrosis), and limb anomalies (hexadactyly, humeral hypoplasia, radial aplasia, and proximally placed thumb). Some patients may have hydrocephalus. Some cases of VACTERL-H are associated with increased chromosome breakage and rearrangement.

Similarity:
Belongs to the GLI C2H2-type zinc-finger protein family.
Contains 5 C2H2-type zinc fingers.

Database links:

Entrez Gene: 7547 Human

Entrez Gene: 22773 Mouse

Entrez Gene: 367944 Rat

Omim: 300265 Human

SwissProt: O60481 Human

SwissProt: Q62521 Mouse

Unigene: 111227 Human

Unigene: 255890 Mouse

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

Product Feedback Wall